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Pulmonary arterial hypertension in congenital heart disease
  1. Paolo Ferrero1,2,
  2. Kaushiga Krishnathasan3,4,
  3. Andrew Constantine3,4,
  4. Massimo Chessa1,2,
  5. Konstantinos Dimopoulos3,4
  1. 1Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, Milan, Italy
  2. 2European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, Rome, Italy
  3. 3Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  4. 4National Heart and Lung Institute, Imperial College London, London, UK
  1. Correspondence to Dr Konstantinos Dimopoulos, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London SW3 6NP, London, UK; k.dimopoulos02{at}

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  • Review the diagnostic pathway for pulmonary arterial hypertension (PAH) in congenital heart disease (CHD).

  • Understand and interpret right heart catheterisation data in pulmonary hypertension related to CHD.

  • Recognise the typical presentation of PAH in patients with CHD.

  • Explore the treatment options for PAH-CHD.

  • Review and understand the management of common emergencies in PAH-CHD.


Pulmonary hypertension (PH) is defined as a mean pulmonary arterial pressure (PAP) above 20 mm Hg, determined by right heart catheterisation. PH is classified into five distinct groups based on underlying pathophysiology and haemodynamics (figure 1).1 2 Patients with pulmonary arterial hypertension (PAH) have precapillary PH haemodynamics, defined as a mean PAP above 20 mm Hg, a pulmonary vascular resistance (PVR) >2 Wood units (WU) and a pulmonary capillary wedge pressure (PCWP) ≤15 mm Hg.2 PAH encompasses conditions with similar pathophysiological characteristics of the pulmonary circulation, and includes PAH associated with congenital heart disease (PAH-CHD), which is the focus of this article (figure 2) 2. PAH affects approximately 10% of patients with CHD, and PAH-CHD accounts for almost a third of the adult PAH population.3 Delays in the diagnosis of PAH can be avoided if patients with CHD are regularly monitored in specialist tertiary centres, where clinicians are vigilant to all complications of CHD.2 4 5

Figure 1

Subgroups of pulmonary hypertension. CHD, congenital heart disease; CTD, connective tissue disease; CTEPH, chronic thromboembolic pulmonary hypertension; CVD, cardiovascular disease; HFpEF, heart failure with preserved ejection fraction; HFrEF, heart failure with reduced ejection fraction; PA, pulmonary artery; PAH, pulmonary arterial hypertension; PCH, pulmonary capillary haemangiomatosis; PH, pulmonary hypertension; PPHN, persistent pulmonary hypertension of the newborn; PVOD, pulmonary veno-occlusive disease. 2

Figure 2

Pulmonary hypertension classification based on right heart catheterisation. ASD, atrial septal defect; Cpc, combined precapillary and postcapillary pulmonary hypertension; mPAP, mean pulmonary artery pressure; PCWP, pulmonary capillary wedge …

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  • PF and KK are joint first authors.

  • PF and KK contributed equally.

  • Contributors KK and PF were responsible for manuscript writing and prepared the manuscript with contributions from all coauthors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

  • Author note References which include a * are considered to be key references.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.